Duodenal gangliocytic paraganglioma derived from neural crest is a peculiar neuroendocrine tumor. It is incidentally found during radiographic studies or due to gastrointestinal hemorrhage caused by frequent ulceration of the overlying mucosa. Most lesions are pedunculated and submucosal with distinctive histology consisting of endocrine cells, ganglion cells and spindle-shaped Schwann cells. We experienced a duodenal gangliocytic paraganglioma in a 40-year-old woman presenting with episodes of melena. Esophagogastroduodenoscopy revealed a submucosal ulcerated tumor in the second portion of duodenum and a biopsy confirmed gangliocytic paraganglioma. The tumor was enucleated through a duodenotomy. It's size was 5.5 X 3 X 1.5 cm sized and revealed positive cellular reaction for chromogranin, synaptophysin, neuron-specific enolase, and neurofilament by immunohistochemistry. The majority of the reported duodenal gangliocytic paraganglioma were of benign nature. Therefore, radical surgery or a lymph node dissection could be avoided if that disease was confirmed. However, thorough investigation for lymph node metastasis and postoperative follow-up are needed. We report the first case of a duodenal gangliocytic paraganglioma in Korea with a review of literature.
Adult ; Duodenal Neoplasms/*diagnosis/pathology/surgery ; English Abstract ; Female ; Humans ; Paraganglioma/*diagnosis/pathology/surgery

Adult ; Humans ; Male ; Paraganglioma ; diagnosis ; surgery ; Seminal Vesicles ; pathology ; surgery ; Tomography, X-Ray Computed

Paraganglioma is a rare neuroendocrine tumor arising from the neural crest, which includes tissues such as the adrenal medulla, carotid and aortic body, organs of Zuckerkandl, and other unnamed paraganglia. The head, neck, and retroperitoneum are the most common sites for paraganglioma. However, paraganglioma of the pancreas is extremely rare. We report our experience of this rare disease. A 70-year old female patient admitted for a pancreas tail mass detected by computed tomography (CT) scan checked for vague left flank pain. CT with contrast enhancement showed a 4.2-cm heterogeneously enhanced lesion in the tail of the pancreas. A well defined ovoid shape mass in left adrenal gland was suggested adenoma. Distal pancreatectomy with left adrenalectomy was performed. Any lymph node enlargement was not found. Pancreas mass did not invade adjacent organ. Microscopic examination with pancreas and adrenal gland revealed that the cells were arranged in a characteristic Zellballen pattern. Immunohistochemical staining revealed positivity for neuron-specific enolase, chromogranin A, synaptophysin, and S-100 protein. On the basis of these findings, we diagnosed the tumor as a paraganglioma of the pancreas and adrenal gland. We report the first case of pancreas paraganglioma in Korea.
Adenoma/diagnosis/surgery ; Adrenal Gland Neoplasms/*diagnosis/secondary/surgery ; Aged ; Chromogranin A/metabolism ; Female ; Humans ; Pancreatic Neoplasms/*diagnosis/pathology/surgery ; Paraganglioma/*diagnosis/secondary/surgery ; Phosphopyruvate Hydratase/metabolism ; S100 Proteins/metabolism ; Synaptophysin/metabolism ; Tomography, X-Ray Computed

Chromogranin A ; metabolism ; Diagnosis, Differential ; Duodenal Neoplasms ; metabolism ; pathology ; surgery ; Ganglioneuroma ; metabolism ; pathology ; Gastrointestinal Stromal Tumors ; metabolism ; pathology ; Humans ; Male ; Middle Aged ; Neurofibroma ; metabolism ; pathology ; Paraganglioma ; metabolism ; pathology ; surgery ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism

Adult ; CD56 Antigen ; metabolism ; Carcinoma ; metabolism ; pathology ; surgery ; Carcinoma, Medullary ; pathology ; Carcinoma, Papillary ; metabolism ; pathology ; DNA-Binding Proteins ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Paraganglioma ; metabolism ; pathology ; Thyroid Neoplasms ; metabolism ; pathology ; surgery ; Thyroidectomy ; methods ; Transcription Factors ; Triglycerides ; metabolism

Adult ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Appendectomy ; Appendiceal Neoplasms ; complications ; metabolism ; pathology ; surgery ; Appendicitis ; etiology ; surgery ; Diagnosis, Differential ; Female ; Granular Cell Tumor ; complications ; metabolism ; pathology ; surgery ; Humans ; Paraganglioma ; metabolism ; pathology ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism

Adult ; Carcinoma, Medullary ; metabolism ; pathology ; Carcinoma, Papillary ; metabolism ; pathology ; Chromogranin A ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Paraganglioma ; metabolism ; pathology ; surgery ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism ; Synaptophysin ; metabolism ; Thyroid Neoplasms ; metabolism ; pathology ; surgery ; Thyroidectomy ; methods

Adult ; Desmin ; metabolism ; Diagnosis, Differential ; Diagnostic Errors ; Humans ; Male ; Melanoma-Specific Antigens ; metabolism ; Meningeal Neoplasms ; metabolism ; pathology ; radiotherapy ; surgery ; Meningioma ; metabolism ; pathology ; Paraganglioma ; metabolism ; pathology ; Sarcoma, Alveolar Soft Part ; metabolism ; pathology ; radiotherapy ; surgery ; Sarcoma, Clear Cell ; metabolism ; pathology ; Tomography, X-Ray Computed ; Vimentin ; metabolism

Adenoma ; metabolism ; pathology ; Adult ; Carcinoma, Renal Cell ; metabolism ; pathology ; Diagnosis, Differential ; Ear Neoplasms ; complications ; genetics ; metabolism ; pathology ; surgery ; Endolymphatic Sac ; Female ; Follow-Up Studies ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; Middle Aged ; Paraganglioma ; metabolism ; pathology ; Point Mutation ; Von Hippel-Lindau Tumor Suppressor Protein ; genetics ; von Hippel-Lindau Disease ; complications ; genetics ; metabolism